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Mitochondrial morphology, bioenergetics and proteomic responses in fatty acid oxidation disorders
Mutations in nuclear genes encoding for mitochondrial proteins very long-chain acyl-CoA dehydrogenase (VLCAD) and trifunctional protein (TFP) cause rare autosomal recessive disorders. Studies in fibroblasts derived from patients with mutations in VLCAD and TFP exhibit mitochondrial defects. To gain...
Autores principales: | Raimo, Serena, Zura-Miller, Gabriella, Fezelinia, Hossein, Spruce, Lynn A., Zakopoulos, Iordanis, Mohsen, Al-Walid, Vockley, Jerry, Ischiropoulos, Harry |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7970426/ https://www.ncbi.nlm.nih.gov/pubmed/33725513 http://dx.doi.org/10.1016/j.redox.2021.101923 |
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